Department of Internal Medicine, The Affiliated Cancer Hospital of Zhengzhou University & Henan Cancer Hospital, Zhengzhou, Henan, 450008, China.
Department of Hematology, The Third People's Hospital of Zhengzhou, Zhengzhou, Henan, 450099, China.
Ann Hematol. 2024 Sep;103(9):3649-3656. doi: 10.1007/s00277-024-05900-y. Epub 2024 Jul 30.
To explore the clinical features, treatment, and prognosis of patients with lymphoma-associated hemophagocytic syndrome (LAHS) in a real-world clinical setting. We retrospectively examined LAHS patients diagnosed at our center between January 2016 and August 2023, focusing primarily on their clinical features, therapeutic approaches, overall response rate (ORR), and overall survival (OS). A combination of univariate and multivariate analyses was conducted to identify potential prognostic factors. A total of 86 patients diagnosed with LAHS were included to evaluate clinical characteristics and prognostic factors. Patients with T/NK cell lymphoma had a higher probability of developing hemophagocytic syndrome (HPS) during the clinical process than those with B cell lymphoma. The median survival time was 55 days for all patients, and 47 and 81 days for the T/NK cell LAHS and B cell LAHS cohorts, respectively (P = 0.025). Among the patients evaluated, the ORR was 42.2%. Patients starting with anti-lymphoma treatment had a better, albeit not significant, ORR than those beginning with anti-HPS treatment. In the univariate analysis, T/NK cell LAHS (P = 0.027), HPS onset at relapse (P = 0.036), higher baseline plasma EBV-DNA levels (> 4,000 copies/mL, P = 0.034), and treatments including cytokine adsorption and ruxolitinib (P < 0.001 and P = 0.017, respectively) were potentially associated with worse OS, while corticosteroid therapy benefited OS. In the multivariate analysis, T/NK cell LAHS (adjusted hazard ratio (aHR) = 2.007), cytokine adsorption therapy (aHR = 4.547), and corticosteroid therapy (aHR = 0.118) were independently associated with mortality. T/NK cell lymphoma was the main cause of LAHS and carried a worse prognosis. Whether anti-lymphoma or anti-HPS treatment should start first still requires prospective studies with larger sample sizes. The key point in controlling HPS is to block the cytokine storm promptly. Corticosteroid therapy is both effective and accessible and should be used early and in sufficient quantities.
为了在真实临床环境中探索淋巴瘤相关噬血细胞综合征（LAHS）患者的临床特征、治疗方法和预后。我们回顾性分析了 2016 年 1 月至 2023 年 8 月在我院诊断为 LAHS 的患者，主要关注其临床特征、治疗方法、总体缓解率（ORR）和总体生存率（OS）。采用单因素和多因素分析来确定潜在的预后因素。共纳入 86 例 LAHS 患者，评估其临床特征和预后因素。与 B 细胞淋巴瘤相比，T/NK 细胞淋巴瘤患者在临床过程中更有可能发生噬血细胞综合征（HPS）。所有患者的中位生存时间为 55 天，T/NK 细胞 LAHS 和 B 细胞 LAHS 队列分别为 47 天和 81 天（P=0.025）。在评估的患者中，ORR 为 42.2%。与开始抗 HPS 治疗的患者相比，开始抗淋巴瘤治疗的患者 ORR 更好，但无统计学意义。单因素分析显示，T/NK 细胞 LAHS（P=0.027）、复发时出现 HPS（P=0.036）、基线血浆 EBV-DNA 水平较高（>4000 拷贝/mL，P=0.034）以及包括细胞因子吸附和鲁索替尼在内的治疗方法（P<0.001 和 P=0.017）与较差的 OS 相关，而皮质类固醇治疗有益于 OS。多因素分析显示，T/NK 细胞 LAHS（调整后的危险比（aHR）=2.007）、细胞因子吸附治疗（aHR=4.547）和皮质类固醇治疗（aHR=0.118）与死亡率独立相关。T/NK 细胞淋巴瘤是 LAHS 的主要原因，预后较差。无论是先进行抗淋巴瘤治疗还是先进行抗 HPS 治疗，仍需要更大样本量的前瞻性研究。控制 HPS 的关键是迅速阻断细胞因子风暴。皮质类固醇治疗既有效又易得，应早期足量使用。
